Longitudinal Assessment of Neuroradiologic Features in Wolfram Syndrome

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FIG 1.
Sagittal, coronal, and axial MR images show pons signal abnormalities as T1 hypointensity and T2 hyperintensity in a patient with Wolfram syndrome (white arrows). Brain stem atrophy is also evident on this MR image.
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FIG 2.
Examples of neuroradiologic findings in patients with Wolfram syndrome. A, Thinning of the optic chiasm (white box) as seen in coronal T1-weighted images (left, patient with Wolfram syndrome; right, healthy control). B, Abnormal PPBS signal (white circles) in midline sagittal T1-weighted images (from left to right, absent, diminished, and physiologic signal). C, White matter hyperintensities in FLAIR MR imaging (white arrowheads).
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FIG 3.
Marked (A) and mild (B) cerebellar atrophy in patients with Wolfram syndrome, as shown in sagittal, coronal, and axial T1-weighted MR images (white arrows), compared with a healthy control (C).
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FIG 4.
A, Frequency of neuroradiologic signs in patients with Wolfram syndrome at first and last visits. B, The relationship between age and the number of neuroradiologic signs. A line connecting a circle-shaped point (first visit) and a triangle-shaped point (last visit) represents each patient.

Table 1:

The demographic and clinical characteristics in patients with Wolfram syndrome at first and last follow-up visits^{^a}

↵a For the age and duration of disease, means and SDs are reported. For comorbid conditions, numbers and percentages are reported.

Table 2:

Neuroradiologic findings in brain MR images in patients with Wolfram syndrome^{^a}

Radiologic Sign	First Visit (n = 30)	Last Visit (n = 30)
Negative PPBS sign^{^b}	16 (53)	21 (70)
T1/T2 pons signal abnormalities	16 (53)	20 (67)
Optic nerve atrophy	9 (30)	24 (80)^{^c}
White matter T2 hyperintensities^{^d}	8 (27)	10 (33)
Cerebellum atrophy	7 (23)	21 (70)^{^c}

↵a Numbers and percentages are reported.
↵b Absent and diminished PPBS sign.
↵c P value < .01 (χ² test).
↵d FLAIR images were not available to evaluate white matter T2 hyperintensity for 1 scan, and T2-weighted images were used instead.